Document Detail

Rituximab in childhood pemphigus vulgaris: a long-term follow-up case and review of the literature.
MedLine Citation:
PMID:  20389028     Owner:  NLM     Status:  MEDLINE    
Pemphigus vulgaris is an infrequent but life-threatening autoimmune blistering disease that is rare in the pediatric age. Although the mainstay of therapy for childhood pemphigus vulgaris (CPV) is steroids, adjuvant immunosuppressive drugs are often needed to control the disease. Thus, an important part of CPV morbidity can be related to treatment. We report the youngest CPV patient described in the English literature, an 18-month-old boy, who has been followed up for 16 years. During this period, the patient has received several immunosuppressive therapies with variable response. He has finally achieved a long-lasting and complete remission with rituximab. Successful treatment with rituximab has previously been reported in 6 cases of CPV, in whom rituximab presented a good side effect profile. Our patient has experienced a chronic and severe clinical course with multiple flares eventually developing vegetative lesions. Since he presented refractoriness to multiple therapies, we administered rituximab. The introduction of this drug led to a dramatic clinical response and a long-term clinical remission. Based on the experience of this case and the data reported in the literature, we believe that rituximab may be a safe and efficacious agent for the treatment of severe CPV.
Irene Fuertes; Antonio Guilabert; Jose Manuel Mascaró; Pilar Iranzo
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2010-04-09
Journal Detail:
Title:  Dermatology (Basel, Switzerland)     Volume:  221     ISSN:  1421-9832     ISO Abbreviation:  Dermatology (Basel)     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-09     Completed Date:  2010-11-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9203244     Medline TA:  Dermatology     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  13-6     Citation Subset:  IM    
Copyright Information:
Copyright 2010 S. Karger AG, Basel.
Department of Dermatology, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain.
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MeSH Terms
Antibodies, Monoclonal, Murine-Derived / therapeutic use*
Antigens, CD20 / blood
Azathioprine / therapeutic use
B-Lymphocytes / drug effects
Child, Preschool
Dapsone / therapeutic use
Desmoglein 1 / blood
Desmoglein 3 / blood
Follow-Up Studies
Gold / therapeutic use
Immunosuppressive Agents / therapeutic use*
Pemphigus / drug therapy*
Prednisone / therapeutic use
Treatment Outcome
Reg. No./Substance:
0/Antibodies, Monoclonal, Murine-Derived; 0/Antigens, CD20; 0/DSG1 protein, human; 0/DSG3 protein, human; 0/Desmoglein 1; 0/Desmoglein 3; 0/Immunosuppressive Agents; 0/rituximab; 446-86-6/Azathioprine; 53-03-2/Prednisone; 7440-57-5/Gold; 80-08-0/Dapsone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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